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Chronic Cryptogenic Sensory Polyneuropathy
Clinical and Laboratory Characteristics
Gil I. Wolfe, MD;
Noel S. Baker, MD;
Anthony A. Amato, MD;
Carlayne E. Jackson, MD;
Sharon P. Nations, MD;
David S. Saperstein, MD;
Choon H. Cha, MD;
Jonathan S. Katz, MD;
Wilson W. Bryan, MD;
Richard J. Barohn, MD
Arch Neurol. 1999;56:540-547.
Background Chronic sensory-predominant polyneuropathy (PN) is a common clinical problem confronting neurologists. Even with modern diagnostic approaches, many of these PNs remain unclassified.
Objective To better define the clinical and laboratory characteristics of a large group of patients with cryptogenic sensory polyneuropathy (CSPN) evaluated in 2 university-based neuromuscular clinics.
Design Medical record review of patients evaluated for PN during a 2-year period. We defined CSPN on the basis of pain, numbness, and tingling in the distal extremities without symptoms of weakness. Sensory symptoms and signs had to evolve for at least 3 months in a roughly symmetrical pattern. Identifiable causes of PN were excluded by history, physical examination findings, and results of laboratory studies. We analyzed clinical and laboratory data from patients with CSPN and compared findings in patients with and without pain.
Results Of 402 patients with PN, 93 (23.1%) had CSPN and stable to slowly progressive PN syndrome. These patients presented with a mean age of 63.2 years and a mean duration of symptoms of 62.9 months. Symptoms almost always started in the feet and included distal numbness or tingling in 86% of patients and pain in 72% of patients. Despite the absence of motor symptoms at presentation, results of motor nerve conduction studies were abnormal in 60% of patients, and electromyographic evidence of denervation was observed in 70% of patients. Results of laboratory studies were consistent with axonal degeneration. Patients with and without pain were similar regarding physical findings and laboratory test abnormalities. Only a few patients (<5%) had no evidence of large-fiber dysfunction on physical examination or electrophysiologic studies. All 66 patients who had follow-up examinations (mean, 12.5 months) remained ambulatory.
Conclusions Cryptogenic sensory polyneuropathy is a common, slowly progressive neuropathy that begins in late adulthood and causes limited motor impairment. Isolated small-fiber involvement is uncommon in this group of patients. Management should focus on rational pharmacotherapy of neuropathic pain combined with reassurance of CSPN's benign clinical course.
From the Departments of Neurology, University of Texas Southwestern Medical Center, Dallas (Drs Wolfe, Baker, Nations, Saperstein, Cha, Katz, Bryan, and Barohn), and University of Texas Health Sciences Center, San Antonio (Drs Amato and Jackson).
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